Congenital Heart Disease and Pregnancy: A Growing Population
Carla Ransom, MD & Jennifer Thompson, MD
Vanderbilt University
12.5.2014
Who are they?
HV: 28 yo G3P0020 at 12 weeks with history of double-inlet LV with left AV valve atresia and transposition of the great arteries, s/p fenestrated lateral tunnel Fontan with residual cyanotic heart disease
PH: 30 yo G1 with history of d-TGA and ASD who is s/p atrial septostomy at birth followed by a Senning atrial baffle
CP: 21 yo G1 with history of subaortic stenosis
Who are they, and where did they all come from?
Past Medical History is Always Important
History of Congenital Heart Surgery (Abbreviated)
1938: Ligation of the Ductus arteriosus
(Dr. Gross, Boston)
1944: Aortic coarctation repair
(Dr. Crafoord, Stockholm)
1944: Blalock-Taussig shunt for Tetralogy of Fallot (Baltimore)
1953: “Heart-Lung Machine” intracardiac repairs
(Dr. Gibbon, Philadelphia)
1954: “Complete” repair of Tetralogy of Fallot
(Dr. Lillehei, Minneapolis)
Controlled Cross-Circulation • A lesson from Maternal Fetal Medicine
• Lillehei March 26, 1954
• VSD closure
Fast Forward
• Oldest congenital heart survivors are in their 6th and 7th decades
• Oldest “complex” survivors are 65 (TOF)
• The surgeries for heart defects changed over time – TOF born in 1946 versus 1992
– D-TGA born in 1965 versus 1980
Childhood Disease • Once fatal diseases have grown into adulthood.
– Cystic fibrosis – 40% of current pts are adult.
– Childhood cancer – 80% survival to adulthood.
• 85% of CHD babies born today reach adult age.
– 60 - 90% mortality reduction from 1979 – 2005.
• Greatest advances in complex disease.
– Patient profile is changing: older and more complex.
JACC 2001;37:1170; Am Heart J 2004;147;425; Heart 2001;85:438; Am Heart J 2009;158:874
Aggregate Survival Changing Severity Distribution
JACC 2001;37:1170
90 95 95
55 65
90
10
50
80
0
20
40
60
80
100
1940-1959 1960-1979 1980-1989
Simple Moderate Complex
% S
urv
ival
to
ad
ult
ho
od
Birth Year
Impact of increasing survival
• More children with severe disease surviving into adulthood
• More women with severe disease are becoming pregnant
• Pregnancy in women with CHD remains a significant cause of morbidity and mortality
Impact of increasing survival
• From 2004-2006, 1.4% of delivery hospitalizations were complicated by chronic heart disease
• There has not been a substantial change in prevalence of chronic heart disease although increases have been seen in women with CHD at the time of delivery
• Increase in severe complications during delivery hospitalizations compared to early time periods
Kukina & Callaghan BJOG 2010
• Number of pediatric congenital heart patients reaching adulthood will continue to increase.
• In Tennessee, estimated 480 moderate-complex CHD patients reach the age of 18 annually.
• Approximately 50% of these are women, many of whom will decide they want to have children and enter into our care
Tennessee’s Epidemiologic Demand Estimates
TN 2012 Adult Population
4.9 million
1% with CHD
49,000
50% with moderate-
complex disease
24,500
TN 2012 Children Population
1.5 million
1% with CHD
15,000
50% with moderate-
complex disease
7,500
CHILDREN <18 years of age ADULTS 18+ years of age
Tennessee’s population: 6,400,000 people
20, 825
Including mortality
• 2010 ACHA Clinic Survey - patients served annually: – UT Memphis 91 – Lexington 110 – Arkansas 300 – Louisville 420 – UAB 430 – Vanderbilt 1,392 – Emory 1,533
• Makes sense that OB care is a reentry point
2010 ACHA Clinic Survey: Self reported
And Not Many ACHD Programs Available in the Southeast
12
Vanderbilt Has Experienced a Decade of Growth
455 495
542 599
642 704
799 893
1016
1147
1392
1599
1869
0
200
400
600
800
1,000
1,200
1,400
1,600
1,800
2,000
2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012
Average Annual Growth
2001-2009: 10% per year
2009-2011: 16% per year
Adult Congenital Heart Patients
Maternal Cardiac Clinic
Vanderbilt has experienced a current trend of 200 new patients per year over the last 5 years
Maternal Cardiology Clinic Disease Mix
Congenital (71%)
Arrhythmia (6.8%)
Cardiomyopathy (6.8%)
Marfan/CTD (6.8%)
Other (8.5%)
General Maternal Cardiology Considerations
• Interplay of two complex systems
• What does the patient know about her disease?
• What is “heart failure” in pregnancy?
• Who can/should get pregnant?
• What can be done to make it successful?
Understanding Why & How Pregnancy can be “Severe”
Evaluation Process • Identify residual lesions
• Determine the baseline functional level
• Recognize the hemodynamic changes of pregnancy and labor and delivery
• Extrapolate from what we know about these lesions in pregnancy – About individual lesions
– About complex lesions (i.e. TOF and Truncus)
– Risk models
Traditional Heart Failure Scoring
• New York Heart Association Classifications
Pregnancy Week =1-10 =11-16 =17-28 =29-40
Heart Failure
• NYHA Functional Classification > 1
– NOT normal for a 35 year old woman
– NORMAL for 35 year old PREGNANT woman?
• Current projects
– Pregnancy specific symptom score
• Not likely to be dyspnea-based (NYHA)
– Normal BNP values in pregnancy
Anticipated Cardiac Changes in Pregnancy
Hemodynamic changes in pregnancy
Plasma Volume Increase up to 40%
Systemic vascular resistance
Decrease 25-30%
Cardiac output Increases 40%
Heart Rate Increases 10-20bpm
Blood pressure Decreases 1st and 2nd trimester Back to normal in 3rd trimester
Central Venous Pressure Unchanged
Nelson-Piercey, Handbook of Obstetric Medicine 2002
Antepartum Physiology
Gabbe: Obstetrics: Normal and Problem Pregnancies, 5th ed.
CO=HR*SV CO 30-50%
HR 10-15 bpm SV 20-30%
Cardiac Output
CO=HR x SV
• CO: 30-50% (peaks 20-24 weeks)
• SV early ( plasma volume and LV dilation)
• HR 10-15 bpm by 32 weeks
Cardiac Output & Maternal Position
• Rolling from LEFT side to BACK will CO by 25%
– Gravid uterus compresses IVC
–Venous return to the heart
–Ventricular filling (SV)
–CO
Physical Findings in Pregnancy
Normal
• Dyspnea
• Syncope
• Palpitations
• Fatigability
• Systolic murmur
Abnormal
• Severe dyspnea
• Syncope with exertion
• Arrhythmias
• Chest pain with exertion
• Diastolic murmur
• Loud, harsh systolic murmur
Normal Imaging Findings
• CXR – Enlarged heart silhouette
• EKG – Mild ST changes in inferior leads
– PACs and PVCs
• Echocardiogram – Tricuspid regurgitation
– LA size increased
– LV outflow cross sectional area increased
Intrapartum Hemodynamics
• Labor – further increase in cardiac output
– Uterine contractions auto-transfusion of 300-500ml of blood back into circulation
• Pain sympathetic response
increase in HR and BP
Postpartum Hemodynamic Changes
• Immediate rise in cardiac output
(Relief of IVC obstruction and uterine contraction)
• Cardiac output increases 60-80% followed by rapid decline to pre-labor values within 1 hour of delivery
• Transfer of fluid from extravascular space increases venous return and stroke volume
Nelson-Piercey, Handbook of Obstetric Medicine 2002
What does this mean for us?
• Minimal data on more complex lesions
– Must extrapolate • Art, not science
• Understand “how the blood goes round”
– Understanding of individual shunts and valve lesions often must be considered in combination
• Of assistance . . .
– General hemodynamic principles
– General risk stratification models
Review of Specific Cardiac Lesions and Severity
Benign in Pregnancy Serious in Pregnancy
Benign Cardiac
Serious Cardiac
ASD, repaired
VSD, repaired
TOF with PS
Atrial Septal Defect
• Opening in the atrial septum allowing blood to shunt from left to right
• Involving septum primum around foramen ovale – secundum ASD
Atrial Septal Defect
• Defects sinus venosus – embryological origin of the junction of SVC and IVC into the right atrium – sinoseptal defects
• Involvement of endocardial cushion tissue – primum ASD
Atrial Septal Defect
• Most common congenital lesion
• Generally asymptomatic
• Tolerate pregnancy, labor and delivery well – Avoid fluid overload
– Vaginal delivered preferred
• 3-10% risk fetal cardiac lesion
Ventricular Septal Defect
• Occur anywhere along the ventricular septum
• Clinical manifestations are determined by size and location
• Defects can be • Inlet • Muscular • Perimembranous • Outlet
Ventricular Septal Defect
• Small defects well tolerated
• Tolerate pregnancy, labor and delivery well UNLESS pulmonary hypertension • Avoid fluid overload • Vaginal delivery
preferred
• 6-10% risk of fetal cardiac
lesion
Benign in Pregnancy Serious in Pregnancy
Benign Cardiac
Serious Cardiac
ASD, repaired
VSD, repaired
TOF with PS
Mitral stenosis
Aortic stenosis
Bicuspid aortic valve
Marfan with normal aorta
Uncorrected TOF
Previous MI
Mitral Stenosis
• Most common rheumatic valve lesion
• Risk varies with severity
• FIXED CARDIAC OUTPUT – LA outflow obstruction LV filling
– LA and pulmonary vascular pressure
• Worsens in pregnancy due to volume
• 25% of patient experience heart failure for the first time in pregnancy
Mitral Stenosis
Mitral Stenosis
• Avoid tachycardia
• Maintain high normal PCWP
– Avoid hypotension
– Avoid fluid overload
• Treat atrial fibrillation
• Epidural strongly recommended
• Shortened second stage
Aortic Stenosis
• Due to bicuspid valve or rheumatic disease
• Worsens due to volume
• FIXED CARDIAC OUTPUT
• Significant if value is 1/3 normal size
Congenital 4-12% risk of fetal lesion
Aortic Stenosis
Aortic Stenosis • Antenatal
– Limit activity
– Growth ultrasound – increased risk of IUGR
– If severe – bedrest consider valve replacement
• Labor
– Maintain high normal PCWP • Avoid hypotension
• Avoid fluid overload
– Epidural slow titration
– Assisted second stage
Uncorrected Tetralogy of Fallot
• Findings
– Pulmonary stenosis
– Right ventricular hypertrophy
– Overriding aorta
– VSD
• Corrected – low risk lesion
• Uncorrected – moderate risk lesion
Uncorrected Tetralogy of Fallot
• 40% incidence of cardiac failure in pregnancy
• Maternal mortality 4-15%
• Fetal mortality 30%
• Risk of IUGR
• Prognosis worse if have pulmonary HTN
5% risk of fetal cardiac lesion
Uncorrected Tetralogy of Fallot
• Avoid hypotension and hypovolemia
– Could cause shunt reversal
• Epidural anesthesia with slow titration
• Assisted second stage
ASD, repaired
VSD, repaired
TOF with PS
Mitral stenosis
Aortic stenosis
Bicuspid aortic valve
Marfan with normal aorta
Uncorrected TOF
Previous MI
Moderate to severe regurgitation
Benign in Pregnancy Serious in Pregnancy
Benign Cardiac
Serious Cardiac
Mitral Regurgitation (MR)
• Facts
– Well-tolerated
– Commonly secondary to mitral valve prolapse
– Longstanding MR ventricular dysfunction or LA enlargement
MR (Cont)
• Workup: – Echo: severity of regurgitation, LV function, LA
enlargement – EKG: A-fib
• Complications:
– Pulmonary edema, arrhythmia (both rare)
• Medical treatment:
– Treatment of arrythmias or decreased EF – Anticoagulation for dilated LA or A-fib
Aortic Regurgitation (AR)
• Facts: – Well-tolerated in pregnancy
– Long-standing AR can LV dysfunction
• Workup with echo to evaluate severity of AR and LA enlargement and EF
• Complications unlikely
• Medical therapy not indicated unless arrhythmia present
ASD, repaired
VSD, repaired
TOF with PS
Mitral stenosis
Aortic stenosis
Bicuspid aortic valve
Marfan with normal aorta
Uncorrected TOF
Previous MI
Moderate to severe regurgitation
Marfan with aortic dilation
Dilated cardiomyopathy
Coarctation of aorta with valve involvement
Fontan/Mustard
Pulmonary HTN
Eisenmenger
Benign in Pregnancy Serious in Pregnancy
Benign Cardiac
Serious Cardiac
Marfan Syndrome
• Defective fibrillin connective tissue weakness
• Increased mortality related to aortic root involvement
• Autosomal dominant
– 50% risk of fetal Marfan syndrome
– Prenatal testing available if mutation is known
Marfan Syndrome
Aortic Root <40mm
• Generally good outcome
• β-blockers decrease pulsatile pressure on aorta
• Epidural
• Vaginal delivery with assisted second stage
Aortic Root >40mm
• Strongly urge pregnancy termination
• β-blockers decrease pulsatile pressure on aorta
• Minimize activity
• Avoid HTN
• Cesarean delivery
• Risk of aortic dissection persists 6-8 wks postpartum
Marfan Syndrome
• Surgical intervention indicated when aortic root diameter of 5cm
• If replacement is due to valvular dysfunction, ascending aortic replacement should be done if diameter is 4.5cm
Cardiomyopathy
• Outcomes depend on degree of LV dysfunction
• Greatest risk in women with EF <45%, prior cardiac event or NYHA class III or IV
• Prior postpartum cardiomyopathy that has improved still at risk of heart failure
• Pregnancy termination should be considered in women with EF <45%
Cardiomyopathy
• Management
– Bedrest
– Na restriction
– Digitalis
– Diuretics
– Consider anticoagulation depending on EF
– Vaginal delivery preferred
Eisenmenger Syndrome HIGHEST RISK LESION
JACC. 2009;53(9):733-740
Eisenmenger Syndrome
• Right left shunting increases in pregnancy due to decreased SVR – Less venous return
– Less pulmonary perfusion
• Maternal mortality 50-75% – Risk highest immediately postpartum to 1 week
postpartum
• Fetal mortality 50% – Increase risk of IUGR
Eisenmenger Syndrome
Antenatal
• Pregnancy termination and sterilization strongly recommended
• Hospitalization
• Serial ultrasounds for growth
• Epoprostoneol or Sildenafil – Improves right sided
pressures by vasodilation
Labor
• PA catheter
• Vaginal delivery with assisted second stage
Single ventricle anomalies
• Tricuspid atresia
• Hypoplastic left heart syndrome
• Double inlet left ventricle
• Heterotaxy defects
• Some double outlet right ventricles
Common surgical correction leads to “Fontan physiology”
Fontan physiology
A SINGLE ventricle is doing the work of pumping oxygenated blood FROM the lungs TO the body while DEOXYGENATED blood returns from the body to the lungs via blood vessel connections only with no pumping chamber in the heart (bypasses the heart).
Fontan complications
Maternal
• Protein Losing Enteropathy; 10%
• Liver or kidney disease
• Arrhythmia
• Leaky valves
• Heart failure
• Thrombosis
• Cyanosis
Fetal
• SAB
• FGR
• Preterm birth
General Hemodynamic Principles
Right heart valve disease
• Obstruction well tolerated
• Regurgitation not as well tolerated
• Example: Tetralogy of Fallot
Left heart valve disease
• Obstruction (even mild) not as tolerable
• Regurgitation well tolerated – drop in SVR and placenta are better afterload
reducers than ACE-Inhibitors
• Example: Bicuspid aortic valve disease
Anemia • Obstructive Diseases, echo gradients worsen • May or may not matter functionally • WILL matter for anesthesia Vascular abnormalities • Marfan • Bicuspid Aortic Valves Cyanosis • Outcomes worsen in relation to lower
saturation • Often due to intracardiac shunts • Increased risk of thromboembolism
Can cardiac risk be stratified during pregnancy?
Existing Risk Stratification Models
• WHO
• CARPREG
• CARPREG + modifications
World Health Organization
• Laundry-list categorizations
• General guide, but can be difficult to extrapolate – extremely heterogeneous population
• Contraceptive guidelines reasonably helpful – more adaptable to broad categorization
– Guidelines – not set in stone! • Weigh quality of life (i.e DUB)
• Weigh risk of cardiac complication to risk of pregnancy
Cardiac Disease in Pregnancy (CARPREG)
Circulation 2001
CARPREG
• 562 prospectively collected pregnant women with heart disease (10/1994-11/1999)
• 13% event rate • Pulmonary edema • Arrhythmia • Stroke • Cardiac Death
• Baseline status was good – 96% were NYHA I or II – 89% were on no cardiac medications
Maternal Morbidity (cont)
• Four predictors for maternal complications:
– 1) Prior cardiac event (heart failure, TIA, stroke, arrhythmia)
– 2) Prepregnancy NYHA class >II
– 3) Left heart obstruction (mitral/aortic valve area <2cm2, peak left outflow gradient >30mm Hg
– 4) EF<40%
Siu et al. 2001
Predictors Defined
• Prior events
– Episode of heart failure
– TIA or CVA
– Arrhythmia
• Poor NYHA class = II or greater
• Cyanosis = resting saturation <88%
Predictors Defined
• Left heart obstruction
– MV area <2cm2
– AV area <1.5cm2
– PEAK echo LVOT gradient >30mmHg
• Reduced LV function
– EF less than 40%
CARPREG Predictor
Predictors Risk of Cardiac Event
0 5%
1 27%
>1 75%
Limitations of CARPREG
• Some limited representation of groups
• Risk index aimed at MOST sensitive assay- captured all events at the expense of specificity!
Circulation 2006
• Modification of CARPREG risk model
• Improved by including
– Decreased subpulmonary ventricular systolic function
– Severe pulmonary regurgitation
• Speaks to the Tetralogy of Fallot population
– Numbers enough to impact outcome numerics (28% of study population)
– Functional status typically good (high representation in reproductive cohorts)
Pulmonary Insufficiency Khairy Circ 2006
Delivery Modifications
Historic Recommendations
• No published systematic reviews on methodology to date
• “Prolonged second stage of labor”
• Increased use of cesarean section in older literature
• Liberal use of invasive monitoring
Toronto Experience
• 559 women with heart disease
• 1118 women without heart disease
• Quartenary care setting
• January 2000-April 2009
• 1677 pregnancies – 594 in 489 women with heart disease
– 35 excluded for TAB, SAB, or multiple gestations.
• 88% were NYHA I, none were NYHA 4
• 5% on anticoagulation
Objectives
1) Compare the peripartum outcomes in women with heart disease to a similar obstetric population, but without heart disease
2) Determine if a less aggressive obstetric management approach, with its benefits, adversely impacts the mother or the neonate.
Types of Cardiac Cases
Congenital (63%)
Valvular (15%)
Arrhythmias (11%)
Cardiomyopathy (7%)
Other (4%)
Congenital by Severity
Mild (24%)
Moderate (59%)
Severe (17%)
Classifications per Connelly M. Canadian Consensus Conference on Adult Congenital Heart Disease. Canadian Journal of Cardiology 1996; 14:395-452.
Modes of Delivery
• Vaginal deliveries result in smaller shifts in blood volume and less hemorrhage
• Fewer overall complications, including infection and thromboembolism
• Increased cardiac workload of labor can be significantly reduced with epidural coverage
Modes of Delivery
• Postpartum hemorrhage was more common in women with heart disease (5% vs. 1%, p< 0.001), but was not related to assisted delivery. The use of invasive (p = 0.05) cardiac monitoring decreased over time. Adverse cardiac events in women with heart disease were rare (2% of pregnancies) and were not associated with mode of delivery or length of the 2nd stage.
Cases Control P-value
Induction of Labor* 232 (50%) 267 (28%) <0.001
Mean GA 38 +/- 2 weeks 38 +/- 3 weeks 0.91
Cesarean Section 182 (33%) 352 (31%) 0.66
Primary 119 (21%) 266 (24%) 0.02
Repeat 63 (11%) 86 (8%) 0.02
Cardiac Indic 2 (0.4%) 0 0.11
Vaginal Deliveries 377 (67%) 766 (69%) 0.5
Assisted Vaginal 164 (29%) 120 (11%) <0.001
Forceps 66 (12%) 27 (2%) <0.001
Vacuum 98 (18%) 93 (8%) <0.001
Cardiac Indic 90 (16%) 0 <0.001
*Includes rates in all vaginal and non-elective CS deliveries; 92% (151/164) after 37 weeks
Modes of Delivery • Despite increased induction rates, C-section rates
no different in two groups
• Assisted delivery is frequent in CHD, but over 50% of women with CHD were able to be delivered spontaneously – many multiparous and precipitous
– others with stable heart disease with no history of cardiac event
• No differences in lacerations despite increased assist rate
Second Stage of Labor
• No significant difference in time of second stage between cases and controls – 55 vs 50 mins, p = 0.18
– Regardless of parity
• If assisted delivery for cardiac indication – Mean duration of second stage was 80 mins
(p<0.001)
– 20% with second stage >190mins (>2SD above control group mean)
Cohort >190mins
• No postpartum hemorrhage requiring transfusion
• No cardiac events
– No correlation between length of second stage and adverse cardiac event
Second Stage, Reconsidered • “Shortened” ≠ “Assisted”
• In some patients, goal is to decrease expulsive effort, not time
• Time for passive descent of presenting part prior to assist
• No differences in cases and controls
– Lacerations
– Fetal trauma
Obstetric Complications
Cases Controls P-values
Postpartum Hemorrhage* 29 (5%) 8 (1%) <0.001
3rd or 4th degree Laceration; Vaginal **
6/292 (3%) 11/748 (2%) 0.50
3rd or 4th degree Laceration; Assisted **
4/90 (4%) 7/49 (14%) 0.04
* Hemorrhage >500ml vaginal or 1L C-section **Lacerations exclude episiotomies
Postpartum Hemorrhage
• 11 women with cardiac disease (2%) required transfusion
• 9 on anticoagulant or were cyanosed
• 2 non-cyanotic women not on anticoagulants • 1 C-section for placenta previa
• 1 with assisted vaginal delivery for heart disease – Not in group with >2SD second stage
Cardiac Events • During labor and delivery and up to 48 hours
postpartum
• None in control group
• Occurred in 2% (8/559) of cardiac cases – CHF (pulmonary edema on CXR or exam), n=5*
– arrhythmia, n=3*
– aortic dissection, n=1
– No cardiac arrest or cardiac death – One event in a vacuum-assisted delivery for cardiac reasons
(second stage = 105 mins)
(*one woman with both a-fib and CHF)
Final Thoughts
• Majority of women with cardiac disease can have successful pregnancy
• Diagnostics prior to pregnancy help in cardiac management during pregnancy (preconception counseling)
• High rate of post-pregnancy interventions
– Many pulmonary valve replacements in TOF
Final Thoughts • Increase in number and severity of CHD
patients to be expected
• Interplay of systems will need interdisciplinary approach – Often to include anesthesia, hematology, genetics
• Understanding of individual anatomy and hemodynamics is essential
• Risk models applied and adjusted to the individual can serve as a guide
Questions?